Metachronous Paget’s disease of the breast: case report

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Introduction

Paget disease, described by James Paget in 1874 (1), is a carcinoma developing from a mammary duct (inva-sive ductal carcinoma), that has a specific affinity to the nipple and areolar skin so that it has been called

“intra-ductal epidermotropic breast cancer” (Tab. 1) (2). The tumor grows along the galattophorous ducts to the are-ola and nipple area producing a typical erythematous, crustous and eczematous-like area that grows into the skin infiltrating the basal layer and looks like hanging drops. The cells contain a lot of mucopolysaccharides that can be recognized microscopically by a surround-ing light halo, thereby distsurround-inguishsurround-ing them from the skin cells. Sometimes the disease is not related to an underlying nodule and in these cases the symptomatol-ogy can feign a dermatologic affection that could lead the physician to treat it in the wrong way for a long time (2).

SUMMARY: Metachronous Paget’s disease of the breast: case

report.

A. GUBITOSI, G. MOCCIA, F.A. MALINCONICO, G. ISIDE, F. GILIO, C. COGNETTI, F. FORONI, G. DOCIMO, R. RUGGIERO, L. DOCIMO, M. AGRESTI

Paget breast disease is a kind of intraductal carcinoma that throu-gh an intracanalicular diffusion invades the basal epidermical layer, reaching the areola and nipple, producing a typical erythematous de-squamative eczematous-like lesion. This neoplasia can remain undetec-ted for a long time and inadequately treaundetec-ted as a dermatological affec-tion. Synchronous or metachronous lesions are very uncommon.

Surgical choice is conditioned by the presence of a tumor below the epidermical lesion, by its dimensions, and by the possible lymph node involvement. Surgical therapy can be radical or conservative.

From our experience we think that lesion biopsy is always necessary to formulate a correct diagnosis and to schedule an appropriate thera-peutic approach. In our case, a biopsy was performed first, then on the basis of the frozen section analysis a radical mastectomy with axillary third level lymph nodes dissection, because of the large dimensions of the lesion and the previous history of a methachronous lesion.

RIASSUNTO: Malattia di Paget metacrona della mammella: caso

clinico.

La malattia di Paget della mammella è un carcinoma intraduttale che attraverso la diffusione intracanalicolare invade lo strato basale epi-dermico, raggiungendo l'areola e il capezzolo e producendo lesioni erite-matose desquamative simileczeerite-matose. La neoplasia può rimanere per lungo tempo misconosciuta e inadeguatamente trattata, spesso come una patologia dermatologica. Lesioni sincrone o metacrone sono molto rare. La scelta chirurgica è condizionata dalla presenza del tumore al di sotto della lesione epidermica, dalle sue dimensioni e dal possibile coin-volgimento linfonodale. La terapia chirurgica può essere radicale o con-servativa.

Dalla nostra esperienza riteniamo che la biopsia della lesione sia sempre necessaria per formulare una corretta diagnosi e programmare un adeguato approccio terapeutico. Nel nostro caso, in primo luogo è stata eseguita una biopsia, quindi, sulla base dell’esame istologico, una mastectomia radicale, con dissezione dei linfonodi ascellari di terzo li-vello, a causa delle grandi dimensioni della lesione e della storia di una pregressa lesione metacrona.

Metachronous Paget’s disease of the breast: case report

G Chir Vol. 30 - n. 4 - pp. 153-155 Aprile 2009

153

Seconda Università degli Studi di Napoli Azienda Ospedaliero-Universitaria

Dipartimento Assistenziale Integrato di Chirurgia Generale

KEYWORDS: Paget disease - Breast cancer - Surgery.

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154

M. Agresti et al.

Case report

A 73-year old woman came to our clinic for an erythematous microulcerated lesion of nipple, areola and periareolar skin of the left breast (Fig. 1). The patient referred that the lesion was seven years old and it had enlarged in the last two months. The patient had undergone a hysterectomy when she was 43 years old and a radical right mastectomy at 61 yrs for Paget-associated ductal car-cinoma (Fig. 2).

Mammography showed a mammary fibroadipose involution

with no focused lesions or microcalcifications, but with the axillary nodes enlarged. US scan confirmed the mammography report. CT scan showed an increase in tissue thickness in the mammary exten-sion up to the axillary area of the left breast (Fig. 3). The bone nuclear study revealed no involvement. Tumoral markers were negative.

The patient underwent skin biopsy that revealed “invasive duc-tal undifferentiated Paget-associated breast carcinoma (pT4b G3)”; immunohistochemical analysis was positive for CK-LMV and CEA, Ki-67 (pos 50%), c-erbB2 (3+).

The patient underwent a radical mastectomy with third level lymph node dissection (Madden). The postoperative course was complication-free and the histology of the specimen confirme the biopsy. The final diagnosis was intraductal carcinoma with residual Paget focus, with metastasis-free regional resected lymph nodes.

Discussion

Paget disease represents 1-4% of all breast cancer (3), appearing frequently in the 5th_-6th _{decade of life}

(4); synchronous (5) and metachronous Paget cases are very rare (6). Diagnosis is reached by clinical

examina-Fig. 1 - Paget’s disease of the left breast.

Fig. 2 - Specimen.

Fig. 3 - Preoperative TC.

TABLE1 - WHO HISTOLOGICAL BREAST CANCER CLAS-SIFICATION.

1. NON INVASIVE

a. Intraductal

b. Lobular carcinoma in situ

2. INVASIVE

a. Invasive ductal carcinoma b. Invasive ductal carcinoma with

a predominant intraductal component c. Invasive lobular carcinoma

d. Mucinous carcinoma e. Medullary carcinoma f. Papillary carcinoma g. Tubular carcinoma h. Adenoid cystic carcinoma i. Secretory (juvenile) carcinoma j. Apocrine carcinoma

k. Carcinoma with metaplasia I. squamous type II. spindle-cell type

III. cartilaginous and osseous type IV. mixed type

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155 tion, cytologic analysis, US scan, mammography, and

CT scan.

Depending on the stadiation, therapeutic options can include radiotherapy, surgery and endocrine therapy (5). Reports in the literature vary widely. Surgical treat-ment is used most frequently; someone use radiotherapy only, others prefer a conservative surgical approach re-serving mastectomy only for large-dimension tumors. In any case, the parameters conditioning therapeutic op-tions are the presence of underlying lesional nodule and its dimensions, enlarged lymph nodes, and hormone re-sponsiveness. Radiotherapy alone is sometimes used for low dimension tumors (< T2) or performed with quad-rantectomy when the lesion is nodule-associated. It can also be used with palliative intent in advanced disease.

Chemotherapy with radiotherapy and hormone therapy can be performed as adjuvant treatments (8).

Surgery can be conservative or radical; quadrantec-tomy, with radio- and/or chemotherapy, is indicated when a palpable nodule is present. In the absence of palpable nodule, a radical mastectomy with axillary dissection should be performed when the lymph nodes are enlarged (9) or, as in our case, when the erythema area is very large. Our surgical choice was conditioned by histological findings of the biopsy (pT4, G3), posi-tive anamnesis for Paget-associated intraductal carci-noma, and axillary lymph node enlargement.

This case report is a simple contribution to an un-common disease, even more rare when we consider the metachronous nature of the lesion.

Metachronous Paget’s disease of the breast: case report

1. Haagensen CD. Malattie e tumori della mammella. Roma: Marrapese ed., 1975

2. Veronesi U, Rabotti GC, Sirtori C. Il carcinoma intraduttale epidermotropo della mammella (cosidetto morbo di Paget). Tumori 1955, supp. 41: 1-142

3. Festi N, Taffurelli M et al. Considerazioni anatomo-cliniche sulla malattia di Paget della mammella. Min. Chir. 1987, 42, 1627-1629

4. Staudacher V, Andreoni B. Manuale di Oncologia Chirurgica. Milano: Masson Italia ed., 1983.

5. Salvadori B, Fariselli B, Saccozzi R. Analysis of 100 cases of Pa-get’s disease of the breast. Tumori 1976; 62: 529-35.

6. Kijima Y, Owaki T, Yoshinaka H, Aikou T. Synchronous bila-teral breast cancer with Paget’s disease and invasive ductal car-cinoma: report of a case. Surg Today. 2003;33(8):606-8. 7. Palade R, Vasilescu D, Grigoriu M. Paget’s disease of a breast–a

special form of a breast cancer. Chirurgia (Bucur). 1995; 44(4):21-7.

8. Sakorafas GH, Blanchard DK, Sarr MG, Farley DR. Paget’s di-sease of the breast: a clinical perspective. Langenbecks Arch Surg. 2001 Nov;386(6):444-50. Epub 2001 Oct 17. 9. Desai DC, Brennan EJ Jr, Carp NZ. Paget’s disease of the

ma-le breast. Am Surg. 1996 Dec;62(12):1068-72.

10. Azzario G, Paino O, Catania S. Il carcinoma di Paget della mammella. Minerva Chir. 1997;52:1389-92.

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